Intravascular large B-cell lymphoma: an elusive diagnosis with challenging management

Mukherjee, Akash; Ibrahim, Ibrahim F.

doi:10.12788/jcso.0425

Cited by 8 publications

(9 citation statements)

References 9 publications

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“… 1 Demonstration of positive expression of CD79a (100%), CD20 (96%), MUM1-IRF4 (95%), CD5 (38%), CD10 (12%), and Myc-positive lymphoma cells within the lumen of blood vessels is diagnostic. 7 , 8 The main clinicopathologic differential diagnosis is that of intravascular natural killer T (T/NK) cell lymphoma, hence search for latent Epstein–Barr virus and immunostaining is important. Considering that skin is commonly involved in the classic or Western variant and cutaneous-only variant, a rapid skin biopsy is key for early diagnosis.…”

Section: Discussionmentioning

confidence: 99%

Intravascular diffuse large B-cell lymphoma with acquired ichthyosis: A case report

Ridha

Zargham

Chergui

et al. 2023

SAGE Open Medical Case Reports

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Intravascular diffuse large B-cell lymphoma is an exceedingly rare subtype of B-cell lymphomas. This cancer is often associated with poor prognosis and can be lethal if left untreated. Intravascular diffuse large B-cell lymphoma is divided into three variants: the ‘classical variant’, the hemophagocytic syndrome-associated variant or ‘Asian variant’, and the ‘cutaneous variant’, according to the clinical presentation and affected organs. We present a unique case of ‘classic variant’ intravascular diffuse large B-cell lymphoma with cutaneous findings, peripheral nervous system involvement and acquired ichthyosis in a patient of Asian descent. This case highlights the importance of a prompt dermatology consultation in the diagnosis of intravascular diffuse large B-cell lymphoma. As bone marrow biopsy is often negative, clinicians must recognize the cutaneous findings and acknowledge that skin biopsy can be an essential tool to establish the diagnosis rapidly. Additional finding making this case unique is the concurrent presence of acquired ichthyosis, which has only been previously reported in one case of intravascular diffuse large B-cell lymphoma.

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Section: Discussionmentioning

confidence: 99%

Intravascular diffuse large B-cell lymphoma with acquired ichthyosis: A case report

Ridha

Zargham

Chergui

et al. 2023

SAGE Open Medical Case Reports

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“…It is characterized by the presence of large lymphoma cells within the lumen of small blood vessels. The absence of cell surface proteins CD29 (B1 integrin) and CD54 (ICAM-1) helps with the diapedesis across the endothelium and the aberrant expression of CD11a and CXCR3 proteins confine lymphoma cells in the intravascular space [2,8]. Based on the geographical origin of the patient, the clinical presentation varies.…”

Section: Discussionmentioning

confidence: 99%

“…Histopathology examination shows large neoplastic lymphoid cells with large nuclei and scant cytoplasm in the blood vessel lumen. Immunophenotypically, besides the strong expression of CD20, it expresses CD79a, MUM1/IRF4, CD5, Pax-5 [1,8].…”

Section: Discussionmentioning

confidence: 99%

Intravascular Large B-Cell Lymphoma: A Diagnostic Dilemma

Roy¹,

Pandey²,

Middleton³

et al. 2021

Cureus

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Intravascular large B-cell lymphoma is a rare malignancy characterized by the presence of lymphoma cells within the lumen of blood vessels. The annual incidence of cases is fewer than 0.5 cases per 1,000,000. It usually affects the elderly with an average age of diagnosis around 70 years. Due to the absence of lymphoma cells in the peripheral smear and lymphadenopathy, it is difficult to diagnose these cases. Although the central nervous system and skin are the commonly involved organs, they can involve any organ system. Prompt diagnosis and initiation of treatment are very crucial as it carries a high mortality. We describe two patients who presented with constitutional symptoms and fever of unknown origin, later diagnosed as intravascular large B-cell lymphoma. The diagnosis was difficult in both cases as the presenting symptoms were atypical. One of the patients was diagnosed at autopsy. The delay in diagnosis often leads to fatal outcomes as the disease is very aggressive. A high degree of clinical suspicion is the key to prompt diagnosis and improved outcomes.

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“…Der wichtigste Unterschied in unserem Fall war die Symptomkonstellation. Intravaskuläre großzellige B‐Zell‐Lymphome, wie sie in der Literatur definiert sind, weisen schwere B‐Symptome und anfängliche Symptome auf, die mit der Tumorlast in Zusammenhang stehen 8–10 . Während unser Fall mit den in der Literatur beschriebenen Fällen insofern übereinstimmt, als die Patientin älter ist, einen schlechten Allgemeinzustand und schwere B‐Symptome aufweist, ist der wichtigste Unterschied das Fehlen einer Organbeteiligung und damit verbundener Symptome 8–10 .…”

Section: Abbildungunclassified

“…Intravaskuläre großzellige B‐Zell‐Lymphome, wie sie in der Literatur definiert sind, weisen schwere B‐Symptome und anfängliche Symptome auf, die mit der Tumorlast in Zusammenhang stehen 8–10 . Während unser Fall mit den in der Literatur beschriebenen Fällen insofern übereinstimmt, als die Patientin älter ist, einen schlechten Allgemeinzustand und schwere B‐Symptome aufweist, ist der wichtigste Unterschied das Fehlen einer Organbeteiligung und damit verbundener Symptome 8–10 . Es gab auch keine Ausbreitung auf das zentrale Nervensystem, das Knochenmark und die hepatosplenische Region, die als die häufigsten Lokalisationen einer IVBCL‐Beteiligung definiert sind.…”

Section: Abbildungunclassified